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Pachydermodactyly - a report of two cases
Zbigniew Żuber1, Grzegorz Dyduch2, Andrzej Jaworek3
1Department of Older Children with subunits of Neurology, Rheumatology and Rehabilitation, St. Louis Regional Specialised Children's Hospital, Krakow, Poland.
Abstract:
Pachydermodactyly (PDD) is a rare and benign form of digital soft tissues fibromatosis, which affects the skin of the fingers. The disorder is characterized by asymptomatic, symmetric, progressive soft tissue swelling of the proximal interphalangeal (PIP) joints of the fingers. The etiology of disease remains unknown. It is usually acquired, even though there are some publications that document family cases. It affects mainly adolescent men. We report two boys with the bilateral swelling of the of the PIP joints of the fingers and skin and subcutaneous tissue thickening. Based on clinical manifestations, radiological study and histopathological examination, pachydermodactyly was diagnosed. PDD is a rare and benign disorder, although it is important to consider other diseases, especially rheumatic conditions, in the differential diagnosis in order to avoid unnecessary additional tests and treatments.
Insights
Pachydermodactyly (PDD) is a rare fibromatosis causing finger swelling. This report details two adolescent males diagnosed with PDD via clinical, radiological, and histopathological evaluation.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Pachydermodactyly (PDD) is a rare, benign fibromatosis affecting digital soft tissues.
- It presents as asymptomatic, symmetric swelling of the proximal interphalangeal (PIP) joints.
- The exact cause is unknown, though it's typically acquired, affecting adolescent males.
Purpose of the Study:
- To report two cases of PDD in adolescent males.
- To highlight the diagnostic process for PDD.
- To emphasize the importance of differential diagnosis from rheumatic conditions.
Main Methods:
- Clinical examination of finger swelling.
- Radiological studies of affected joints.
- Histopathological examination of tissue samples.
Main Results:
- Two adolescent males presented with bilateral PIP joint swelling and tissue thickening.
- Diagnosis of PDD was confirmed through combined clinical, radiological, and histopathological findings.
- The condition was benign and asymptomatic.
Conclusions:
- Pachydermodactyly is a rare, benign condition requiring careful diagnosis.
- Differential diagnosis is crucial to exclude rheumatic diseases and prevent unnecessary interventions.
- Early and accurate diagnosis aids in appropriate patient management.
